Bile composition in Alagille Syndrome and PFIC patients having Partial External Biliary Diversion.
BACKGROUND
Partial External Biliary Diversion (PEBD) is a surgical intervention to treat children with Progressive Familial Intrahepatic Cholestasis (PFIC) and Alagille syndrome (AGS). PEBD can reduce disease progression, and examining the alterations in biliary lipid composition may be a prognostic factor for outcome.


METHODS
Biliary lipid composition and the clinical course of AGS and PFIC patients were examined before and after PEBD.


RESULTS
Pre-PEBD bile from AGS patients had greater chenodeoxycholic/cholic acid (CDCA/CA), bile salt, cholesterol and phospholipid concentrations than PFIC patients. AGS patients, and PFIC patients with familial intrahepatic cholestasis 1 (FIC1) genotype, responded better to PEBD than PFIC patients with bile salt export protein (BSEP) genotype. After successful PEBD, AGS patients have higher biliary lipid concentrations than PFIC patients and PEBD also increases biliary phospholipid concentrations in FIC1 patients.


CONCLUSIONS
Both AGS and FIC1 patients can benefit from PEBD, and preserved biliary phospholipid concentrations may be associated with better outcomes post-PEBD.
Emerick
Karan M
KM
Children's Memorial Hospital, Chicago, IL, USA. kemerick@ccmckids.org
Elias
Marc S
MS
Melin-Aldana
Hector
H
Strautnieks
Sandra
S
Thompson
Richard J
RJ
Bull
Laura N
LN
Knisely
A s
As
Whitington
Peter F
PF
Green
Richard M
RM
D000293:Adolescent; D016738:Alagille Syndrome / Q000235:genetics / Q000378:metabolism*; D001646:Bile / Q000378:metabolism*; D001653:Bile Ducts, Intrahepatic / Q000601:surgery; D001706:Biopsy; D002635:Chenodeoxycholic Acid / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D002780:Cholestasis, Intrahepatic / Q000235:genetics / Q000378:metabolism*; D002784:Cholesterol / Q000378:metabolism; D019826:Cholic Acid / Q000378:metabolism; D013505:Digestive System Surgical Procedures / Q000379:methods*; D018450:Disease Progression; D005704:Gallbladder / Q000601:surgery; D006801:Humans; D007223:Infant; D050356:Lipid Metabolism / Q000502:physiology*; D008099:Liver / Q000473:pathology; D010743:Phospholipids / Q000378:metabolism*; D016896:Treatment Outcome
D010743:Phospholipids; D002635:Chenodeoxycholic Acid; D002784:Cholesterol; D019826:Cholic Acid